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Angiosarcoma Management- Understanding a Rare Vascular Cancer

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Angiosarcoma is a cancer that can appear anywhere on the body. The article below briefs in detail about it.

Written by

Hemamalini. R

Medically reviewed by

Dr. Rajesh Gulati

Published At November 2, 2023
Reviewed AtNovember 2, 2023

What Is Angiosarcoma?

Angiosarcoma is a cancer that can appear anywhere in the body. It affects the inner lining of blood vessels. The disease most frequently manifests in the skin, breast, liver, spleen, and deep tissues. Angiosarcoma is most frequently found on the scalp and face; most cases are cutaneous angiosarcomas. It forms beneath the skin's surface and is called subcutaneous angiosarcomas. 25% of angiosarcomas occur in deep tissue, and 8% of these tumors occur in breast tissue.

Angiosarcomas can occur in men and women of any race, but they rarely do in kids. Angiosarcoma patients can benefit from seeking treatment at a specialized cancer center where they can access a proficient sarcoma team and dedicated resources, ensuring they receive expert care promptly.

What Is the Cause of Angiosarcoma?

A sarcoma typically has an unknown cause. Lymphedema, the swelling of a body part due to fluid accumulation, is the most well-known cause of angiosarcoma. Angiosarcoma has been linked to carcinogens like vinyl chloride, arsenic, and thorium dioxide, and it can also develop due to radiation exposure or treatment.

How Does Angiosarcoma Develop?

Although it can be challenging to prove, scientists constantly try to understand how cancer develops. Some of the risk factors for developing angiosarcoma are as follows:

  • Lymphedema is when too much lymph fluid accumulates in the tissues, resulting in swelling, typically in the arms or legs.

  • Radiation therapy in the past, such as treatment for breast cancer or Hodgkin lymphoma.

  • Exposure to some chemicals that cause cancer.

Is Angiosarcoma Inherited?

Angiosarcoma can run in families. Angiosarcoma of the heart can occur in people who have a change in the POT1 gene, and this change can be passed on to the children of those individuals.

What Are the Stages of Angiosarcoma?

The stage of angiosarcoma is determined by two key factors: the size of the tumor and whether it has metastasized to nearby lymph nodes or distant areas of the body. If the tumor has not spread, it is said to be localized. Metastatic angiosarcoma indicates the tumor has spread beyond nearby tissues to the lungs or other organs.

What Are the Symptoms of Angiosarcoma?

Angiosarcoma can manifest in numerous ways. It may resemble a skin infection, a bruise, or a nonhealing lesion. One should be especially concerned if such an area appears at a site of prior radiation therapy because it could be violet in color. It could also manifest as a visible or palpable soft lump. Deep-seated tumors might go undetected until they affect the tissues and organs around them.

What Are The Risk Factors Associated With Angiosarcoma?

Angiosarcoma is a rare cancer, and its exact causes are unknown. However, several risk factors have been identified, including previous radiation therapy, chemical exposure (such as vinyl chloride or arsenic), chronic lymphedema, certain genetic conditions, and potential associations with viruses, autoimmune diseases, and genetic mutations. It is important to remember that having these risk factors does not guarantee the development of angiosarcoma.

How to Diagnose Angiosarcoma?

The following tests and procedures are used to diagnose angiosarcoma:

  • Physical Exam: Healthcare providers will examine a patient to determine the condition.

  • Biopsy: Obtaining a tissue sample for examination. Doctors may take a sample of the suspect tissue for testing in a lab. The procedure is referred to as a biopsy. Lab tests can be used to find cancer cells. Specialized tests allow a physician to gain more knowledge about cancer cells.

  • Imaging Tests: Imaging examinations aid physicians in assessing cancer's scope and severity. A few possible tests include MRI, CT, and positron emission tomography (PET). The tests you take will depend on the circumstances.

What Is the Treatment Plan For Angiosarcoma?

The best angiosarcoma treatment will depend on the situation. The medical team considers the cancer's size, location, and whether it has spread to other body parts. Treatment choices could be:

  • Surgery: The entire angiosarcoma will be removed during surgery. The surgeon will perform a procedure to remove the cancerous cells and certain portions of the neighboring healthy tissue. In certain situations, surgery may not be a viable choice. This may occur if the cancer has spread to other parts of the body or is very large.

  • Radiation Therapy: In radiation therapy, high-energy beams such as X-rays and protons eliminate cancer cells. After surgery, radiation therapy may be used to eradicate any cancer cells that remain. If surgery is not an option for you, radiation therapy may be.

  • Chemotherapy: Chemotherapy is a medical procedure used to eradicate cancer cells. If the angiosarcoma has spread to other parts of the body, chemotherapy may be an option. If surgery is not an option, chemotherapy, and radiation therapy may occasionally be combined.

  • Targeted Drug Therapy: Targeted drug treatments attack specific chemicals in cancer cells. By blocking these chemicals, targeted drug treatments can cause cancer cells to die. Targeted drugs might be an option for angiosarcoma treatment if the cancer is advanced.

  • Immunotherapy: Immunotherapy fights cancer by activating the immune system. Because cancer cells make proteins that help them hide from immune system cells, the body's immune system may not attack the cancer. Immunotherapy works by obstructing that action. Immunotherapy may be a viable option for treating advanced cancer in the case of angiosarcoma.

What Are The Considerations For Follow-up Care After Treatment?

After completing treatment, regular follow-up visits with the healthcare team are crucial for monitoring the condition and detecting any signs of recurrence or potential complications. The frequency of follow-up visits and the specific tests performed may vary depending on individual circumstances.

What Is the Prognosis for Angiosarcoma Patients?

Angiosarcoma patients frequently experience a poor prognosis due to a late diagnosis that occurs after the disease has already spread throughout the body. According to studies, patients with smaller tumors removed with clean margins tend to have better outcomes. Breast angiosarcoma of low grade has a better prognosis.

What About Clinical Research Trials in Angiosarcoma?

Treatment for angiosarcoma can be difficult. In certain situations, the doctor might advise participating in a clinical trial. Imaging tests performed later on may be used to track how the cancer is responding to therapy.

Conclusion:

After angiosarcoma treatment, patients should be closely monitored. Follow-up appointments are recommended every three months for the first two years, then every six months for the next three years to ensure well-being and detect potential issues. Generally, angiosarcoma patients feel lonely. Members of the family are the main support for such patients. So, they need to help the patients feel comfortable by encouraging them, changing their thinking, staying with them for a longer time, providing valuable advice for their future, and creating a supportive environment with calm.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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